| Autor: |
Sophie Tezenas du Montcel, Perrine Charles, Pascale Ribai, Cyril Goizet, Alice Le Bayon, Pierre Labauge, Lucie Guyant-Maréchal, Sylvie Forlani, Celine Jauffret, Nadia Vandenberghe, Karine NGuyen, Isabelle Le Ber, David Devos, Carlo-Maria Vincitorio, Mario-Ubaldo Manto, François Tison, Didier Hannequin, Merle Ruberg, Alexis Brice, Alexandra Durr |
| Předmět: |
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| Zdroj: |
Brain: A Journal of Neurology; May2008, Vol. 131 Issue 5, p1352-1352, 1p |
| Abstrakt: |
Reliable and easy to perform functional scales are a prerequisite for future therapeutic trials in cerebellar ataxias. In order to assess the specificity of quantitative functional tests of cerebellar dysfunction, we investigated 123 controls, 141 patients with an autosomal dominant cerebellar ataxia (ADCA) and 53 patients with autosomal dominant spastic paraplegia (ADSP). We evaluated four different functional tests (nine-hole pegboard, click, tapping and writing tests), in correlation with the scale for the assessment and rating of cerebellar ataxia (SARA), the scale of functional disability on daily activities (part IV of the Huntington disease rating scale), depression (the Public Health Questionnaire PHQ-9) and the EQ-5D visual analogue scale for self-evaluation of health status. There was a significant correlation between each functional test and a lower limb score. The performance of controls on the functional tests was significantly correlated with age. Subsequent analyses were therefore adjusted for this factor. The performances of ADCA patients on the different tests were significantly worse than that of controls and ADSP patients; there was no difference between ADSP patients and controls. Linear regression analysis showed that only two independent tests, the nine-hole pegboard and the click test on the dominant side (P $$\begin{array}{c}\multicolumn{1}{c}{\mathrm{CCFS}=\mathrm{log}{}_{10}(7+\frac{Z\hspace{0.17em}\mathrm{pegboard}\hspace{0.17em}\mathrm{dominant}\hspace{0.17em}\mathrm{hand}}{10}+4\times \frac{Z\hspace{0.17em}\mathrm{click}\hspace{0.17em}\mathrm{dominant}\hspace{0.17em}\mathrm{hand}}{10})}\end{array}$$ The CCFS score was significantly higher in ADCA patients compared to controls (1.12 ± 0.18 versus 0.85 ± 0.05, Pc Pc P Pc P < 0.0001). The pegboard and click tests are easy to perform and accurately reflect the severity of the disease. The CCFS is a simple and validated method for assessing cerebellar ataxia over a wide range of severity, and will be particularly useful for discriminating paucisymptomatic carriers from affected patients and for evaluating disease progression in future therapeutic trials. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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